Dia­gno­sis

Lupus is a rare dise­a­se with diver­se, often non-spe­ci­fic sym­ptoms. It can take years to reach a cor­rect diagnosis. 

Joint pain or inflamma­ti­on is the initi­al sym­ptom in 60% of affec­ted indi­vi­du­als, promp­ting them to seek medi­cal attention.

Dia­gno­sing Syste­mic Lupus Ery­the­ma­to­sus (SLE) can be chal­len­ging, as not all clas­si­fi­ca­ti­on cri­te­ria are always met at the onset.

Skin chan­ges are the first sym­ptom in appro­xi­m­ate­ly 20% of affec­ted individuals.

Diver­se Sym­ptoms Com­pli­ca­te Diagnosis

Gene­ral sym­ptoms such as seve­re fati­gue and fever are often pre­sent con­curr­ent­ly and are the pri­ma­ry initi­al sym­ptom in 5–10% of affec­ted individuals. 

In 10–15% of affec­ted indi­vi­du­als, SLE beg­ins with the invol­vement of inter­nal organs such as the kid­neys or the ner­vous system.

It is cru­cial that Syste­mic Lupus Ery­the­ma­to­sus is dia­gno­sed as quick­ly as pos­si­ble to ensu­re opti­mal and time­ly tre­at­ment for affec­ted individuals.

Sym­ptoms and Labo­ra­to­ry Values

The detec­tion of spe­ci­fic anti­bo­dies in the blood – anti­nu­clear anti­bo­dies (ANA), anti-dsDNA anti­bo­dies, anti-Smith anti­gen anti­bo­dies, and anti­car­dio­li­pin anti­bo­dies – can aid in diagnosis.

Other labo­ra­to­ry values also con­tri­bu­te to the dia­gno­sis of SLE: inflamma­to­ry mar­kers, decrea­sed com­ple­ment fac­tors (C3, C4), reduc­tion in leu­ko­cytes, lym­pho­cytes, or plate­lets, etc.

Exami­na­ti­on of kid­ney func­tion para­me­ters, inclu­ding uri­na­ly­sis, can also pro­vi­de indi­ca­ti­ons of SLE.

During a medi­cal con­sul­ta­ti­on, it is important for affec­ted indi­vi­du­als to pro­vi­de pre­cise infor­ma­ti­on about all their symptoms.

SLE dia­gno­sis typi­cal­ly invol­ves: Cli­ni­cal exami­na­ti­on by the phy­si­ci­an, gui­ded by SLE dia­gno­stic cri­te­ria. Labo­ra­to­ry tests, which include spe­ci­fic auto­an­ti­bo­dy determination. 

SLE Dia­gno­stic Criteria

The dia­gno­sis of SLE is based on the ACR/​EULAR clas­si­fi­ca­ti­on criteria. 

The new ACR and EULAR clas­si­fi­ca­ti­on cri­te­ria for lupus from 2019 include posi­ti­ve ANA anti­bo­dies as an ent­ry cri­ter­ion for lupus diagnosis.

Cli­ni­cal Criteria

Con­sti­tu­tio­nal Symptoms
Points
Fever
2
Skin
Points
Non-scar­ri­ng alopecia
2
Oral ulcers
2
Subacu­te cuta­neous or dis­co­id LE
4
Acu­te cuta­neous LE
6
Arthri­tis
Points
Syn­ovi­tis in ≥ 2 joints or ten­der­ness in ≥ joints with mor­ning stiff­ness ≥ 30 min
6
Neu­ro­lo­gy
Points
Deli­ri­um
2
Psy­cho­sis
3
Sei­zu­res
5
Sero­si­tis
Points
Pleu­ral or peri­car­di­al effusion
5
Acu­te pericarditis
6
Hema­to­lo­gy
Points
Leu­ko­pe­nia
3
Throm­bo­cy­to­pe­nia
4
Auto­im­mu­ne hemolysis
4
Kid­neys
Points
Pro­te­in­uria ≥ 0.5 g/​24h
4
Lupus nephri­tis (histol.) Type II, V
8
Lupus nephri­tis (histol.) Type III, IV
10

Immu­no­lo­gi­cal Criteria

Anti­phos­pho­li­pid-AK
Points
Anti­car­dio­li­pin IgG ≥ 40 GPL
2
Anti-β2G­P1 IgG ≥ 40 GPL
2
Lupus anti­co­agu­lant
2
Com­ple­ment
Points
C3 or C4 decreased
3
C3 and C4 decreased
4
Auto-AK
Points
Anti-dsDNA-AK
6
Anti-Smith-AK
6

Clas­si­fi­ca­ti­on as SLE ANA ≥ 1:80 and ≥ 10 points

ACR/​EULAR cri­te­ria 2019 for the clas­si­fi­ca­ti­on of syste­mic lupus ery­the­ma­to­sus by: Arin­ger M.: EULAR/​ACR clas­si­fi­ca­ti­on cri­te­ria for SLE. Semin Arthri­tis Rhe­um 2019; 49, S14-S17